Acylcarnitines

Interconversion of acyl-CoA esters and acylcarnitines plays a key role in
the transport process of fatty acids across the mitochondrial membrane.
Acylcarnitine profiling in plasma has become indispensable for the
diagnosis of a series of fatty acid ß-oxidation deficiencies, and of
defects specifically related to the membrane transport process.
Increasingly, acylcarnitine profiling in blood spots by MS/MS is included
in national neonatal screening programs